Freeman Roy
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA.
Semin Neurol. 2003 Dec;23(4):435-42. doi: 10.1055/s-2004-817727.
Orthostatic hypotension is the most incapacitating symptom of autonomic failure. This disorder occurs with both central autonomic neurodegenerative disorders, such as multiple system atrophy and Parkinson's disease, and peripheral autonomic disorders, such as the autonomic peripheral neuropathies and pure autonomic failure. The hallmark of both central and peripheral causes of neurogenic orthostatic hypotension is the failure to release norepinephrine appropriately upon standing. Patient education is the cornerstone of management. There are several measures that can be implemented to improve orthostatic tolerance prior to pharmacological intervention. Plasma volume expansion is essential to improve orthostatic tolerance, and fluid and sodium chloride intake should be increased. Most patients can be treated successfully with volume expansion or fludrocortisone or both in combination with a sympathomimetic agent. Desmopressin acetate and erythropoietin are useful supplementary agents in patients with more refractory symptoms. There are rare patients who will require additional agents to treat their symptoms. A small group of patients remain refractory to all therapeutic modalities.
直立性低血压是自主神经功能衰竭最使人丧失能力的症状。这种病症在中枢自主神经退行性疾病(如多系统萎缩和帕金森病)以及外周自主神经疾病(如自主神经外周神经病和单纯自主神经功能衰竭)中都会出现。神经源性直立性低血压的中枢和外周病因的共同特征是站立时不能适当地释放去甲肾上腺素。患者教育是管理的基石。在进行药物干预之前,可以采取几种措施来提高直立耐受性。扩充血浆容量对于提高直立耐受性至关重要,应增加液体和氯化钠的摄入量。大多数患者可以通过扩充容量或使用氟氢可的松或两者与拟交感神经药联合治疗而成功治愈。醋酸去氨加压素和促红细胞生成素对症状更难治疗的患者是有用的辅助药物。有极少数患者需要额外的药物来治疗其症状。一小部分患者对所有治疗方式均无反应。